Niels Vejlstrup1, Keld Sørensen2, Eva Mattsson2, Ulf Thilén2, Per Kvidal2, Bengt Johansson2, Kasper Iversen2, Lars Søndergaard2, Mikael Dellborg2, Peter Eriksson2. 1. From Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (N.V., L.S.); Skejby Hospital, Aarhus, Denmark (K.S.); Department of Cardiology, Karolinska University Hospital, Stockholm, Sweden (E.M.); Department of Cardiology, Skåne University Hospital, Lund University, Sweden (U.T.); Department of Cardiology, University Hospital of Uppsala, Sweden (P.K.); Department of Public Health and Clinical Medicine, Umeå University, Sweden (B.J.); GUCH Center Göteborg, Department of Cardiology, Herlev County Hospital, Denmark (K.I.); and Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Göteborg, Sweden (M.D., P.E.). niels.vejlstrup@regionh.dk. 2. From Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (N.V., L.S.); Skejby Hospital, Aarhus, Denmark (K.S.); Department of Cardiology, Karolinska University Hospital, Stockholm, Sweden (E.M.); Department of Cardiology, Skåne University Hospital, Lund University, Sweden (U.T.); Department of Cardiology, University Hospital of Uppsala, Sweden (P.K.); Department of Public Health and Clinical Medicine, Umeå University, Sweden (B.J.); GUCH Center Göteborg, Department of Cardiology, Herlev County Hospital, Denmark (K.I.); and Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Göteborg, Sweden (M.D., P.E.).
Abstract
BACKGROUND: The atrial switch operation, the Mustard or Senning operation, for the transposition of the great arteries (TGA) was introduced in the late 1950s and was the preferred surgery for TGA until the early 1990s. The Mustard and Senning operation involves extensive surgery in the atria and leaves the right ventricle as the systemic ventricle. The Mustard and Senning cohort is now well into adulthood and we begin to see the long-term outcome. METHODS AND RESULTS: All the 6 surgical centers that performed Mustard and Senning operations in Sweden and Denmark identified all operated TGA patients. Information about death was obtained in late 2007 and early 2008 from the Danish and Swedish Centralised Civil Register by using the patients' unique national Civil Registration Numbers. Four hundred sixty-eight patients undergoing the atrial switch operation were identified. Perioperative 30-day mortality was 20%, and 60% were alive after 30 years of follow-up. Perioperative mortality was significantly increased by the presence of a ventricular septal defect, left ventricular outflow obstruction, surgery early in the Mustard and Senning era. However, only pacemaker implantation is predictive of long-term outcome (hazard ratio, 1.90; 95% confidence interval, 1.05-3.46, P=0.04), once the TGA patient has survived the perioperative period. The risk of reoperation was correlated to the presence of associated defects and where the first Mustard/Senning operation was performed. CONCLUSIONS: The long-term survival of patients with Mustard and Senning correction for TGA appears to be primarily determined by factors in the right ventricle and tricuspid valve and not the timing of or the type of surgery in childhood. Cardiac function necessitating the implantation of a pacemaker is associated with an increase in mortality.
BACKGROUND: The atrial switch operation, the Mustard or Senning operation, for the transposition of the great arteries (TGA) was introduced in the late 1950s and was the preferred surgery for TGA until the early 1990s. The Mustard and Senning operation involves extensive surgery in the atria and leaves the right ventricle as the systemic ventricle. The Mustard and Senning cohort is now well into adulthood and we begin to see the long-term outcome. METHODS AND RESULTS: All the 6 surgical centers that performed Mustard and Senning operations in Sweden and Denmark identified all operated TGA patients. Information about death was obtained in late 2007 and early 2008 from the Danish and Swedish Centralised Civil Register by using the patients' unique national Civil Registration Numbers. Four hundred sixty-eight patients undergoing the atrial switch operation were identified. Perioperative 30-day mortality was 20%, and 60% were alive after 30 years of follow-up. Perioperative mortality was significantly increased by the presence of a ventricular septal defect, left ventricular outflow obstruction, surgery early in the Mustard and Senning era. However, only pacemaker implantation is predictive of long-term outcome (hazard ratio, 1.90; 95% confidence interval, 1.05-3.46, P=0.04), once the TGA patient has survived the perioperative period. The risk of reoperation was correlated to the presence of associated defects and where the first Mustard/Senning operation was performed. CONCLUSIONS: The long-term survival of patients with Mustard and Senning correction for TGA appears to be primarily determined by factors in the right ventricle and tricuspid valve and not the timing of or the type of surgery in childhood. Cardiac function necessitating the implantation of a pacemaker is associated with an increase in mortality.
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Authors: Odilia I Woudstra; Tjitske E Zandstra; Rosanne F Vogel; Arie P J van Dijk; Hubert W Vliegen; Philippine Kiès; Monique R M Jongbloed; Anastasia D Egorova; Pieter A F M Doevendans; Thelma C Konings; Barbara J M Mulder; Michael W T Tanck; Folkert J Meijboom; Berto J Bouma Journal: J Am Heart Assoc Date: 2021-02-22 Impact factor: 5.501